Pharmacotherapy for Idiopathic Pulmonary Arterial Hypertension During the Past 25 Years

Abstract

Recently developed pharmacotherapies offer greater effectiveness and safety than traditional agents for the treatment of IPAH.

It's all about sex: gender, lung development and lung disease

“…Idiopathic pulmonary arterial hypertension (IPAH), a pulmonary vasculopathy of unknown etiology, predominantly affects females [22]. Recent therapies such as epoprostenol, bosentan and sildenafil have been directed at the arterial vascular bed to induce vasodilatation and reduce pulmonary vascular resistance.…”

Successful Bosentan and Nonnucleoside Reverse Transcriptase Inhibitor‐Based Therapy in a Patient with Acquired Immunodeficiency Syndrome and Pulmonary Arterial Hypertension

“…2C8/9 (weak), 2C19 (weak), recommended 18 ; when given in increased cGMP results in 2D6 (weak), 2E1 (weak), combination with etravirine, pulmonary vasculature and 3A4 (weak) 13,18 there is a 57% decrease in relaxation; vasodilation in the sildenafil's AUC, and the pulmonary bed and the sildenafil dose may need to be systemic circulation (to a increased based on clinical effect 20 lesser degree) may occur 13 Mycobacterium tuberculosis, avium, and xenopi when she was diagnosed with AIDS. At that time, her CD4 + count was 10 cells/mm 3 (3%) (normal range 800-1200 cells/mm 3 ) and HIV viral load was greater than 500,000 copies/ml.…”

“…The symptoms of PAH are typically insidious in onset and include progressive dyspnea, lethargy, and fatigue. Due to the nonspecific nature of these symptoms, diagnosis of PAH in and is also an inducer of result in a 5-fold increase in endothelium and smooth CYP3A4 and 2C9; auto-bosentan trough concentration; muscle leading to reductions induction of metabolism ritonavir is a potent inhibitor in vasoconstriction and may occur; multiple doses of OATP and CYP3A4; patients vascular remodeling [13][14][15] of bosentan may decrease who have received ritonavir plasma concentrations to for at least 10 days should 50-65% after single-dose start bosentan at a dosage of administration 14,15 62.5 mg once/day or every other day; patients receiving bosentan should discontinue the drug at least 36 hrs before starting ritonavir; after at least 10 days, bosentan should be resumed at 62.5 mg once/day or every other day based on tolerability Ambrisentan…”

Physicians’ and patients’ expectations of therapies for pulmonary arterial hypertension: where do they meet?

“…The disease is heterogeneous and, although idiopathic PAH is the most common diagnosis in registries [3][4][5], PAH can also be heritable, induced by drugs or toxins, or associated with conditions such as connective tissue disease, congenital heart disease, portal hypertension, HIV infection or schistosomiasis [6]. Initial diagnosis can be difficult as patients often present with non-disease-specific symptoms such as breathlessness, fatigue, peripheral oedema, chest pain during exertion, light headedness, syncope and abdominal distension [7,8].…”